June 2019 is HHT Awareness Month

I’ve been a nose bleeder for as long as I can remember.  I have vague memories as a young boy with my parents holding towels up to my nose trying to get the bleeding to stop.  I remember a specific instance my freshman year of high school of a major nosebleed during gym that was something out of a slasher horror film.  Its wasn’t until years later that I was diagnosed with an inherited disease that causes malformed blood vessels called HHT (Hereditary Hemorrhagic Telangiectasia) also known as Osler-Weber-Rendu syndrome.

It is amazing the things in life that we will suffer through.  Things that we have learned to incorporate into our daily lives, work around, or just try to avoid instead of attempting to find the root cause and eliminate the problem once and for all.  I was no different with my constant nosebleeds.  Watch a movie, get a nosebleed.  Stand in the checkout line, get a nosebleed.  Sit in a business meeting, get a nosebleed.  In the middle of the night, get a nosebleed. 

I have years of evidence of my nosebleeds.  I’ve lost count the number of times that I have run out of the room with no warning to others.  The ruined shirts and pillow cases.  Blood marks on the seatbelts of my vehicles.  The bathroom sink that looks like it should be a crime scene investigation. 

I don’t remember what my breaking point was.  I don’t recall the exact moment where I said enough was enough and I had to get a grip on this.  Back in 2007, I finally reached that limit.  And it was good that I did, as things were going to get whole lot scarier.

I scheduled an appointment with a local ear, nose and throat doctor or otolaryngologist (ENT).  I remember the first visit distinctly.  A quick scope of my nose and he said “hmm, that’s strange”.  Instead of the normal tree branch shape of blood vessels, he described it as little pods.  But I underwent the typical treatment for a nosebleed which was a chemical cauterization of the surface level blood vessels with powdered silver nitrate.

Let me explain the silver nitrate procedure really quickly.  Both nostrils are hit with a numbing spray and the silver nitrate is put on a something like a Q-tip.  It burns, cauterizing the vessels.  I had two main side effects of this treatment.  The worst running nose you can possibly imagine that lasted 12-24 hours.  And once the numbing spray wore off, the sensation was if someone lit a match inside my skull.  It wasn’t fun.

I thought the problem was solved. And it was.  For about a year.  Then the nosebleeds came back.

We repeated the silver nitrate treatment a couple more times.  Each time with less of a result.  Finally, my ENT recommended an outpatient electrical cauterization procedure at the hospital.  The main difference I experienced with this procedure is my nose was packed with a gel like substance that was supposed to dissolve in a couple of days.

But the gel didn’t dissolve.  It hardened like concrete and was extremely uncomfortable. 

I got an emergency follow up with my ENT who had to use the equivalent of medical needle nose plyers to pull out the now solidified gel packed in each of my nostrils.  Again, not fun.

This triggered a decent nosebleed in the office.  He chose to treat it with more silver nitrate, on the open wound, without numbing spray.  It was like a full bonfire going on in my head.

My ENT leaves me in the room for roughly an hour.  And he comes back and says I need to setup an appointment at the Johns Hopkins Hospital HHT Center of Excellence.  He thought I had this rare disease that he hasn’t encountered before called HHT. 

At this point we are in 2011, nearly 4 years since my first trip to the ENT.  I wasn’t my normal self.  Friends of mine were noticing how I seemed out of breath doing mundane activities.  Watching TV, I would sit and sweat.  I was bowling and if you saw me it looked like I had just run a marathon. I was worn out all the time.  I didn’t know it at the time, but the fatigue and the shortness of breath turned out to be another symptom of HHT, but in my lungs.

The time for my testing and appointments at Johns Hopkins finally came around mid-2011.  It was at these appointments where I finally understood the potential gravity of HHT and my situation.  I was given an expansive list of tests:  brain MRI, chest CT scan, heart echo, EKG and blood work.  Also, physical exams from multiple doctors. The Johns Hopkins team is great.

The doctors were looking for arteriovenous malformations (AVM) in my brain and in my lungs.  These are abnormal blood vessels that form in people with HHT.  They disrupt normal blood flow and oxygen circulation.  They can have abnormal structures or pool up blood.  And I had several in my lung.  Including a big one in my lungs that resembled a small water balloon filled with blood.  My oxygen saturation level was in the 80s.  This explained the crazy sweating and being out of breath.  I was at risk of having a stroke or having my newly discovered AVM burst and hemorrhage.

My procedure to fix the AVM was scheduled within a couple days of the diagnosis. It was in an older building at Johns Hopkins back then.  The prep room and the surgical rooms were divided by the family waiting room.  They ran out of gurney’s right before my procedure.  I threw on another gown, so my rear wasn’t hanging out and walked thru the waiting room like a boss.  And jumped on the table myself.

The procedure to treat this from the patient prospective is relatively simple.  A catheter goes up through the groin and it’s completely painless.  It is similar in nature to angioplasty, but the catheter goes into the lungs. 

I won’t ever forget the first time I sat up after the procedure.  It was like getting a shot of adrenaline.  My O2 level was back up in the high 90s.   I felt fantastic.

There are some areas too small to be treated.  I just completed my 3-year checkup (May 2019) and will have to undergo the procedure again.  One of the vessels has grown to the point it needs to be dealt with. 

I am fortunate at this stage in life to be on the lesser end of the severity scale.  I currently sit between an 1-2 on the 10-point nosebleed severity chart.  I must remember to take antibiotics before dentist appointments as I am more susceptible to a blood infection.  That is easier to prevent than to treat.

We have installed a whole home humidifier to help with the dry air during the winter months.  It has greatly reduced the amount of horror film style nose bleeds that I used to get when the dry heated air is cranking full blast during the cold months.

I participated in Johns Hopkins double blind clinical trial a few years ago.  They were testing out different sprays to help with nosebleeds.  I get a follow up call from that once a year.  They check in to see how I am doing.

I will be monitored for the rest of my life.  I am crossing my fingers that as I get older, the progression of HHT in my GI tract, liver, etc. will be on the minor side.  But I do expect something.  I just turned 40 and I get to experience my first colonoscopy before my next lung treatment later this summer.  Joy.

I urge everyone, if you know someone that has regular consistent nosebleeds to go see visit your nearest HHT Center of Excellence or local ENT if you are not near one. Ask your local ENT if they are aware of HHT.  If you don’t have nosebleeds, but your oxygen level is lower than a 97%, ask to get checked.  Don’t let an aversion to doctors prevent you from getting a test that could save your life.  It is estimated that 85%-90% of people are currently undiagnosed.

I never got my nose bleed problem solved. And probably never will.  But I know that finally getting checked prevented me from experiencing a potentially deadly outcome.

Don’t wait!

For further information please visit https://curehht.org.  The Cure HHT Foundation website contains information about HHT Centers of Excellence, Physicians, and other resources. 

You will find a donate button, where 93 cents of every dollar funds scientific research, drive awareness campaigns, and help create Centers of Excellence.

John Barker

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